21-03-2013 дата публикации
Номер: US20130072473A1
Принадлежит:
The present invention is directed to compounds of Formulae (I), (IIa-IIh), (IIIa-IIIe), (IVa-IVc), (Va-V1), (VIa-VII), (VII), (VIII) and (IX), pharmaceutical compositions thereof and methods of use thereof in the treatment of conditions associated with a dysfunction in proteostasis. 112-. (canceled)1427-. (canceled)28. The method of claim 13 , wherein Dis N(R) and wherein each Ris independently selected from the group consisting of hydrogen and optionally substituted C-Calkyl.2932-. (canceled)33. The method of claim 28 , wherein Gis an optionally substituted 5/6-membered fused heteroaryl.34. The method of claim 33 , wherein Gis benzothiazolyl claim 33 , benzoxazolyl claim 33 , benzimidazolyl claim 33 , benzothiophenyl claim 33 , and benzofuranyl claim 33 , each optionally substituted.3646-. (canceled)47. The method of claim 35 , wherein Gis an optionally substituted 5/6-membered fused heteroaryl.48. The method of claim 47 , wherein Gis benzothiazolyl claim 47 , benzoxazolyl claim 47 , benzimidazolyl claim 47 , benzothiophenyl claim 47 , and benzofuranyl claim 47 , each optionally substituted.5065-. (canceled)70. The method of claim 13 , wherein the condition is associated with a dysfunction in the proteostasis of a protein selected from the group consisting of hexosamine A claim 13 , cystic fibrosis transmembrane conductance regulator claim 13 , aspartylglucsaminidase claim 13 , a-galactosidase A claim 13 , cysteine transporter claim 13 , acid ceremidase claim 13 , acid α-L-fucosidase claim 13 , protective protein claim 13 , cathepsin A claim 13 , acid β-glucosidase claim 13 , acid β-galactosidase claim 13 , iduronate 2-sulfatase claim 13 , α-L-iduronidase claim 13 , galactocerebrosidase claim 13 , acid α-mannosidase claim 13 , acid β-mannosidase claim 13 , arylsulfatase B claim 13 , arylsulfatase A claim 13 , N-acetylgalactosamine-6-sulfate sulfatase claim 13 , acid β-galactosidase claim 13 , N-acetylglucosamine-1-phosphotransferase claim 13 , acid sphingmyelinase ...
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